Rhodotorula spp. are emergent opportunistic pathogens, particularly in immunocompromised individuals. They have been associated with endocarditis, peritonitis, meningitis endophthalmitis and catheter-associated fungemia. The aim of this study was to review all cases of central venous catheter-related fungemia due to Rhodotorula spp. reported in the literature in order to determine the best management of this uncommon infection. All patients but one in the 88 cases examined had some form of underlying disease including sixty-nine (78.4%) who had cancer. Rhodotorula mucilaginosa was the species most frequently recovered (75%), followed by Rhodotorula glutinis (6%). Amphotericin B deoxycholate was the most common antifungal agent used as treatment and the overall mortality was 9.1% in this review. This fungemia is a rare disease which can be found in immunocompromised and in the intensive care patients. The use of specific antifungal therapy may be associated with an increase in the survival. It should be noted that Rhodotorula spp. is resistant to fluconazole.